Pemphigus Foliaceus

General Information
Types of Pemphigus
Diagnosis
Treatment
Who Gets Pemphigus?
Caregiving

General Information

Pemphigus is a group of rare autoimmune blistering diseases of the skin and/or mucous membranes.

Our immune system produces antibodies that normally attack hostile viruses and bacteria in an effort to keep us healthy. In a person with pemphigus, however, the immune system mistakenly perceives the cells in skin and/or mucous membrane as foreign, and attacks them. Antibodies that attack one's own cells are called autoantibodies. The part of the cells that are attacked in pemphigus are proteins called desmogleins. Desmogleins form the glue that attaches adjacent skin cells, keeping the skin intact.

When auto-antibodies attack desmogleins, the cells become separated from each other. The skin virtually becomes unglued. This causes burn-like lesions or blisters that do not heal. In some cases, these blisters can cover a significant area of the skin.

Types of Pemphigus

There are three main categories of pemphigus: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus,

Pemphigus is not pemphigoid, cicatricial pemphigoid or benign familial pemphigus, also known as Hailey-Hailey disease.

Pemphigus Vulgaris (PV)

The term "vulgar" means "common," and PV is the most frequently diagnosed form of pemphigus. Sores and blisters almost always start in the mouth. Because the skin is an organ, PV is called a one-organ disease. It does not affect any of the internal organs. The blisters can go as far down as the vocal cords, but no further. PV does not cause permanent scars unless there is infection associated with the lesion.

In PV, auto-antibodies attack the protein “glue,” which holds skin cells together, called desmogleins. The lesions are painful. Sometimes there is the Nikolsky effect where just touching the skin can cause it to tear. Before drug treatment, PV was 99% fatal, but today, with the current therapies, the mortality rate is only 5 to 15%.

Pemphigus Foliaceus (PF)

In pemphigus foliaceus, blisters and sores do not occur in the mouth. Crusted sores or fragile blisters usually first appear on the face and scalp and later involve the chest and back.

Auto-antibodies are produced by the immune system but they bind only to desmoglein 1. The blisters are superficial and often itchy, but are not usually as painful as PV.

In PF, disfiguring skin lesions can occur, but the mortality rate from the disease is much lower than in PV.

Paraneoplastic Pemphigus (PNP)

PNP is the most serious form of pemphigus. It occurs most often in someone who has already been diagnosed with a malignancy (cancer). Fortunately, it is also the least common. Painful sores of the mouth, lips and esophagus are almost always present; and skin lesions of different types occur. PNP can affect the lungs. In some cases, the diagnosis of the disease will prompt doctors to search for a hidden tumor. In some cases the tumor will be benign and the disease will improve if the tumor is surgically removed.

It is important to know that this condition is rare and looks different than the other forms of pemphigus. The antibodies in the blood are also different and the difference can be determined by laboratory tests.

Diagnosis

Because it is rare, pemphigus is often the last disease considered during diagnosis. Consult a dermatologist if there are any persistent skin or mouth lesions. Early diagnosis may permit successful treatment with only low levels of medication.

There are three criteria that must be met for a definite diagnosis:

Proper clinical presentation — visual examination of skin lesions.
Lesion biopsy — A sample of the blistered skin is removed and examined under the microscope to determine if the cells are separated in the manner characteristic of pemphigus. Additionally, the layer of skin in which cell-to-cell separation occurs can be determined.
Direct immunoflourescence — The biopsy skin sample is treated to detect desmoglein antibodies in the skin. The presence of these antibodies indicates pemphigus.

In addition to the above, another diagnostic test that may be used is called indirect immunofluorescence or antibody titer test. This measures desmoglein autoantibodies in the blood serum. It may be used to obtain a more complete understanding of the course of the disease. In addition, a serum assay for desmoglein antibodies, known as ELISA, is also available. It is the most accurate but, unfortunately, is not available in all clinical labs.

Treatment

Treatment for pemphigus vulgaris (PV) involves the use of one or more drugs. Initially, PV is treated with a corticosteroid. Other drugs are usually used in conjunction with corticosteroids. These medications can have serious side effects. Patients on these medications must have blood and urine monitored on a regular basis. There is some evidence suggesting that treatment is easier in the early stages of the disease.

Who Gets Pemphigus?

To date, definitive statistics on the incidence and prevalence of pemphigus are not available, but estimates of the number of new cases diagnosed each year ranges from as high as 5 per one hundred thousand to as low as one per million, depending upon the type of pemphigus and the ethnicity of the affected population. It is known to affect people across racial and cultural lines. However, there are certain groups of people (such as eastern European Jews and people of Mediterranean descent) who have a higher incidence of the disease. Men and women are equally affected. Research studies suggest a genetic predisposition to the disease. Although the onset usually occurs in middle-aged and older adults, PV and PF also occur in young adults and children.

Pemphigus is frequently the last disease considered during diagnosis. If you have any persistent skin or mouth lesions, consult your dermatologist. Early diagnosis may permit treatment with low levels of medication.

Caregiving

Though the concerns of those who live with pemphigus are considerable, the impact on spouses, family members, and caregivers should not be ignored or underestimated. Many caregivers take advantage of the online discussion group not only in behalf of the person for whom they care, but to get information, assistance, and even support from other caregivers in similar circumstances. If you are a caregiver, please feel free to use the online discussion group as a tool to help in your endeavors, as well as to contribute your knowledge and experience to those who might need you help.

Being a caregiver comes with its own set of challenges. The uncertainty of the disease, and the need many caregivers feel to always appear to be strong and positive often creates stresses and difficulties which make the challenge even greater. One of the most helpful tools in dealing with this issue is to become as informed as possible. When your loved one has questions, it is of great comfort to him or her to be able to hear a good answer. In general, there is very much to be positive about regarding treatment and control of pemphigus. Understanding the basics of the disease, along with general treatment protocol, as well as having a few anecdotal instances gives you and your loved one the assurance that you are not alone and that what you are experiencing has been experienced and overcome by someone else.

Source: International Pemphigus Organization

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